骨化性湿疹--老年霍奇金淋巴瘤的一种皮肤表现

EJHaem Pub Date : 2024-06-26 DOI:10.1002/jha2.910
Ariane Le Clainche Compagnie, Charlotte Degoutte, Barbara Papouin, Claire Lamaison, Romain Gounot, Saskia Ingen-Housz-Oro
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引用次数: 0

摘要

一名 82 岁的男性因体重下降 13 公斤和红斑演变 2 个月而被转诊。临床检查发现,患者的躯干主要出现骨化性湿疹(AE)(图 1A),并伴有掌跖角化病和颈上多发性腺病。从生物学角度看,患者表现为 11 g/dL 非再生性正常血细胞性贫血、12.4 G/L 中性粒细胞多核细胞增多症、0.4 G/L 淋巴细胞减少症和 21 g/L 低白蛋白血症。影像学检查(计算机断层扫描[CT]和正电子发射断层扫描(PET)-CT)证实了膈上高代谢多腺瘤病。皮肤活检显示鱼鳞状皮肤病,提示为副肿瘤性过程(图1C)。结节活检确诊为典型的霍奇金淋巴瘤(图 1D)。患者在前两次灌注中接受了单用布伦妥昔单抗维多汀的治疗,获得了AE临床完全缓解(CR)(没有PET-CT再评估),随后联合使用了pembrolizumab进行强化治疗,对淋巴瘤和皮肤病均有效,四个周期后获得了CR。由于 CR 和免疫毒性(肾病和胰腺炎),在使用了四剂 brentuximab vedotin 和两剂 pembrolizumab 后,治疗停止。一名 78 岁的男性因伴有掌跖角化症的弥漫性 AE 而住院治疗(图 1B)。临床检查时发现右侧腹股沟有一个 3 厘米长的单个坚实无痛腺病。CT 扫描显示原始和外部右髂腺病。结节活检结果与 Epstein-Barr 病毒阴性的典型霍奇金淋巴瘤一致。AE于1907年由Bocq首次描述,是一种影响老年患者的特殊形式的皮肤干燥症,由皮脂腺和汗腺分泌枯竭引起[1]。它可能存在于健康人身上,但某些表型特征,如深层炎性裂纹,以躯干为主,应超过可能的潜在肿瘤。AE 与血液病之间的关联已有描述,尤其是在非霍奇金淋巴瘤中[2]。与典型霍奇金淋巴瘤的关联则较为罕见。17%-53%的典型霍奇金淋巴瘤患者有皮肤表现,主要表现为非特异性病变,可能是疾病的门户[3]。最常见的皮肤表现是瘙痒(20%的病例)。其他表现还包括多形性皮肤症状,如瘙痒症、副肿瘤性丘疹、多形性红斑、结节性红斑和各种糜烂(牛皮癣、湿疹和银屑病)。临床医生应根据 AE 的表现识别 AE,并意识到 AE 可能揭示霍奇金淋巴瘤,尤其是在老年患者中。
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Asteatotic eczema, a cutaneous manifestation of Hodgkin lymphoma in older patients

An 82-year-old man was referred for a 13 kg weight loss and an erythroderma evolving for 2 months. Clinical examination revealed an asteatotic eczema (AE) predominant on the trunk (Figure 1A) associated with palmoplantar keratoderma and supracentimetric cervical polyadenopathy. Biologically, the patient presented with 11 g/dL nonregenerative normocytic anemia, neutrophilic polynucleosis of 12.4 G/L, lymphopenia of 0.4 G/L, and hypoalbuminemia of 21 g/L. Imaging (computed tomography [CT] scan and positron emission tomography (PET)-CT) confirmed supradiaphragmatic hypermetabolic polyadenopathy. Skin biopsy showed ichthyosiform dermatosis suggestive of a paraneoplastic process (Figure 1C). Node biopsy confirmed the diagnosis of classic Hodgkin lymphoma (Figure 1D). The patient was treated with brentuximab vedotin alone for the first two perfusions which permitted AE clinical complete remission (CR) (no PET-CT reevaluation was available) and then combined with pembrolizumab for treatment intensification which was effective on both the lymphoma and the dermatosis, and which permitted a CR after four cycles. Treatment was stopped after four doses of brentuximab vedotin and two doses of pembrolizumab because of CR and immunomediated toxicities (nephropathy and pancreatitis). He is still in CR after 10 months of follow-up.

A 78-year-old man was hospitalized for a diffuse AE associated with palmoplantar keratoderma (Figure 1B). A single firm and painless right inguinal adenopathy measuring 3 cm was found at clinical examination. The CT scan showed primitive and external right iliac adenopathies. Node biopsy was consistent with Epstein-Barr virus-negative classic Hodgkin lymphoma. The patient died suddenly of unknown cause before beginning the treatment.

AE, first described in 1907 by Bocq, is a particular form of xerosis affecting older patients, which results from a depletion of the secretion of the sebaceous and sweat glands [1]. It may be present in healthy individuals, but certain phenotypic features such as deep, inflammatory cracks with trunk predominance should outweigh possible underlying neoplasia. The association between AE and hemopathies is already described, especially in non-Hodgkin lymphoma [2]. The association with classic Hodgkin lymphoma is rarer. Skin manifestations are present in 17%–53% of patients with classic Hodgkin lymphoma, mainly characterized by non-specific lesions, and can be the disease's gateway [3]. The most frequent skin manifestation is pruritus (20% of cases). Other manifestations include polymorphic skin symptoms, such as prurigo, paraneoplastic pemphigus, erythema multiforme, erythema nodosum, and various kinds of eruptions (bullous, eczematous, and psoriatic). Clinicians should recognize AE by its presentation and be aware that it may reveal Hodgkin lymphoma, especially in older patients.

The authors declare no conflict of interest.

No funding has been received for this article.

The authors have confirmed ethical approval statement is not needed for this submission.

We have patient consent for this plubication in patient consent statement.

The authors have confirmed clinical trial registration is not needed for this submission.

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